Zur Diagnostik des Diabetes insipidus unter besonderer Berücksichtigung hypophysektomierter Patienten

Verschiedene, am gleichen Patienten durchgeführte Stimulationsteste für die ADH-Sekretion (17-Std.-Durstversuch, Carter-Robbins-Test, osmotische Diurese) zeigen eine ausgezeichnete Korrelation. Auf Grund dieser Ergebnisse werden Richtlinien zur möglichst einfachen Diagnostik einer Konzentrationsstörung vorgeschlagen. In den meisten Fällen kann allein schon auf Grund eines exakt durchgeführten Durstversuchs eine Konzentrationsstörung nachgewiesen (höchste Urinosmolalität 750 mOsm/kg) werden. Lediglich bei einer höchsten Urinosmolalität im 17-Std.-Durstversuch zwischen 500 und 750 mOsm/kg müssen aufwendigere und den Patienten mehr belastende Tests (z. B. Carter-Robbins-Test) eingesetzt werden, um das Vorliegen bzw. den Grad einer Konzentrationsstörung endgültig diagnostizieren zu können. ADH-Injektionen dienen dabei zur Differenzierung zwischen einem ADH-Mangel und einer mangelhaften ADH-Ansprechbarkeit der Niere. Unsere Untersuchungen an hypophysektomierten Patienten (N=29) zeigen auch bei Patienten ohne polyurisch-polydiptisches Syndrom (N=22) eine gegenüber Normalpersonen signifikant eingeschränkte Konzentrationsleistung im 17-Std.-Durstversuch.The results of three different stimulation tests for ADH (17 hours thirst period, Carter-Robbins-test, osmotic diuresis) correlate well in the same patient. Because of this we propose a simplified procedure for the detection of failure to produce a concentrated urine. In most cases merely on the basis of a precisely executed thirst period test, one can either recognize an abnormality of concentration (highest urine osmolality less than 500 mOsm/kg), or exlude it (highest urine osmolality greater than 750 mOsm/kg). Only when the highest urine osmolality lies between 500 and 750 mOsm/kg after a 17 hour thirst must more painstaking tests (e.g. Carter-Robbins test) be employed to determine both the presence of and the degree of a failure to produce a concentrated urine. An injection of ADH can be used to differentiate between a deficiency of ADH and a defective response of the kidneys to ADH. Our investigations on hypophysectomized patients (n=29) showed that also in patients without the syndrome of polyuria-polydypsia (n=22), there was a significantly reduced capacity to concentrate urine during a 17 hour thirst period, as compared with normal people

Intra-operative MRI facilitates tumour resection during trans-sphenoidal surgery for pituitary adenomas

Background During trans-sphenoidal microsurgical resection of pituitary adenomas, the extent of resection may be difficult to assess, especially when extensive suprasellar and parasellar growth has occurred. In this prospective study, we investigated whether intra-operative magnetic resonance imaging (iMRI) can facilitate tumour resection. Methods Twenty patients with macroadenomas, (16 non-functioning, three growth-hormone secreting and one pharmaco-resistant prolactinoma) were selected for surgery in the iMRI. The mean tumour diameter was 27 mm (range 11–41). The mean parasellar grade, according to the Knosp classification, was 2.3. Pre-operative coronal and sagittal T1-weighted and T2-weighted images were obtained. The trans-sphenoidal tumour resection was performed at the edge of the tunnel of a Signa SP 0.5-Tesla MRI. The surgeon aimed at a radical tumour resection that was followed by a peri-operative MRI scan. When a residual tumour was visualised and deemed resectable, an extended resection was performed, followed by another MRI scan. This procedure was repeated until the imaging results were satisfactory. In all patients, we were able to obtain images to assess the extent of resection and to classify the resection as either total or subtotal. Results After primary resection, eight out of 20 cases were classified as total resections. A second resection was performed in 11 of 12 cases classified as subtotal resections, and in four of these, total resection was achieved. A third resection was performed in three of the remaining seven cases with subtotal resections, but we did not achieve total resection in any of these cases. Therefore, the use of iMRI increased the number of patients with total resection from 8/20 (40%) to 12/20 (60%). The only observed complication was a transient spinal fluid leakage. Conclusion Intra-operative MRI during trans-sphenoidal microsurgery is useful in selected patients for a safe and more complete resection. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited

Acromegaly

Acromegaly is an acquired disorder related to excessive production of growth hormone (GH) and characterized by progressive somatic disfigurement (mainly involving the face and extremities) and systemic manifestations. The prevalence is estimated at 1:140,000–250,000. It is most often diagnosed in middle-aged adults (average age 40 years, men and women equally affected). Due to insidious onset and slow progression, acromegaly is often diagnosed four to more than ten years after its onset. The main clinical features are broadened extremities (hands and feet), widened thickened and stubby fingers, and thickened soft tissue. The facial aspect is characteristic and includes a widened and thickened nose, prominent cheekbones, forehead bulges, thick lips and marked facial lines. The forehead and overlying skin is thickened, sometimes leading to frontal bossing. There is a tendency towards mandibular overgrowth with prognathism, maxillary widening, tooth separation and jaw malocclusion. The disease also has rheumatologic, cardiovascular, respiratory and metabolic consequences which determine its prognosis. In the majority of cases, acromegaly is related to a pituitary adenoma, either purely GH-secreting (60%) or mixed. In very rare cases, acromegaly is due to ectopic secretion of growth-hormone-releasing hormone (GHRH) responsible for pituitary hyperplasia. The clinical diagnosis is confirmed biochemically by an increased serum GH concentration following an oral glucose tolerance test (OGTT) and by detection of increased levels of insulin-like growth factor-I (IGF-I). Assessment of tumor volume and extension is based on imaging studies. Echocardiography and sleep apnea testing are used to determine the clinical impact of acromegaly. Treatment is aimed at correcting (or preventing) tumor compression by excising the disease-causing lesion, and at reducing GH and IGF-I levels to normal values. Transsphenoidal surgery is often the first-line treatment. When surgery fails to correct GH/IGF-I hypersecretion, medical treatment with somatostatin analogs and/or radiotherapy can be used. The GH antagonist (pegvisomant) is used in patients that are resistant to somatostatin analogs. Adequate hormonal disease control is achieved in most cases, allowing a life expectancy similar to that of the general population. However, even if patients are cured or well-controlled, sequelae (joint pain, deformities and altered quality of life) often remain

Endocrinologic, neurologic, and visual morbidity after treatment for craniopharyngioma

Craniopharyngiomas are locally aggressive tumors which typically are focused in the sellar and suprasellar region near a number of critical neural and vascular structures mediating endocrinologic, behavioral, and visual functions. The present study aims to summarize and compare the published literature regarding morbidity resulting from treatment of craniopharyngioma. We performed a comprehensive search of the published English language literature to identify studies publishing outcome data of patients undergoing surgery for craniopharyngioma. Comparisons of the rates of endocrine, vascular, neurological, and visual complications were performed using Pearson’s chi-squared test, and covariates of interest were fitted into a multivariate logistic regression model. In our data set, 540 patients underwent surgical resection of their tumor. 138 patients received biopsy alone followed by some form of radiotherapy. Mean overall follow-up for all patients in these studies was 54 ± 1.8 months. The overall rate of new endocrinopathy for all patients undergoing surgical resection of their mass was 37% (95% CI = 33–41). Patients receiving GTR had over 2.5 times the rate of developing at least one endocrinopathy compared to patients receiving STR alone or STR + XRT (52 vs. 19 vs. 20%, χ2P < 0.00001). On multivariate analysis, GTR conferred a significant increase in the risk of endocrinopathy compared to STR + XRT (OR = 3.45, 95% CI = 2.05–5.81, P < 0.00001), after controlling for study size and the presence of significant hypothalamic involvement. There was a statistical trend towards worse visual outcomes in patients receiving XRT after STR compared to GTR or STR alone (GTR = 3.5% vs. STR 2.1% vs. STR + XRT 6.4%, P = 0.11). Given the difficulty in obtaining class 1 data regarding the treatment of this tumor, this study can serve as an estimate of expected outcomes for these patients, and guide decision making until these data are available

ICAR: endoscopic skull‐base surgery

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